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https://www.readbyqxmd.com/read/27911337/effect-of-the-butyrate-prodrug-pivaloyloxymethyl-butyrate-an9-on-a-mouse-model-for-spinal-muscular-atrophy
#1
Jonathan D Edwards, Matthew E R Butchbach
Spinal muscular atrophy (SMA) is an early-onset motor neuron disease that leads to loss of muscle function. Butyrate (BA)-based compounds markedly improve the survival and motor phenotype of SMA mice. In this study, we examine the protective effects of the BA prodrug pivaloyloxymethyl butyrate (AN9) on the survival of SMNΔ7 SMA mice. Oral administration of AN9 beginning at PND04 almost doubled the average lifespan of SMNΔ7 SMA mice. AN9 treatment also increased the growth rate of SMNΔ7 SMA mice when compared to vehicle-treated SMNΔ7 SMA mice...
November 29, 2016: Journal of Neuromuscular Diseases
https://www.readbyqxmd.com/read/27911308/the-effects-of-gene-mutations-on%C3%A2-default%C3%A2-mode-network-in%C3%A2-familial%C3%A2-alzheimer-s-disease
#2
Xiaozhen Li, Eric Westman, Steinunn Thordardottir, Anne Kinhult Ståhlbom, Ove Almkvist, Kaj Blennow, Lars-Olof Wahlund, Caroline Graff
Familial Alzheimer's disease (FAD) mutations have very high penetrance but age at onset and rate of disease progression differ. Neuroimaging and cerebrospinal fluid (CSF) examinations in mutation carriers (MCs) may provide an opportunity to identify early biomarkers that can be used to track disease progression from presymptomatic to the dementia stages of disease. The default mode network (DMN) is a resting state neuronal network composed of regions known to associate with amyloid deposition in AD. We hypothesized that functional connectivity in the DMN might change at pre-clinical stages in FAD MCs and correlate with changes in CSF biomarkers as a consequence of AD brain pathology...
November 29, 2016: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/27910123/congenital-infantile-fibrosarcoma-mimicking-a-cutaneous-vascular-lesion-a-case-report-and-review-of-the-literature
#3
Tyler Enos, Gregory A Hosler, Naseem Uddin, Adnan Mir
Congenital infantile fibrosarcoma (CIFS) is a rare neoplasm of infancy that occurs most frequently in the extremities, and when presenting in the skin, may sometimes resemble infantile hemangiomas or other vascular lesions. Clinically, these tumors differ from hemangiomas in the time of onset, morphology, and growth pattern and must be evaluated histologically for definitive diagnosis. We describe an infant with a neoplasm involving the distal left forearm initially presumed to be a vascular lesion after evaluation by two separate ultrasound studies...
November 7, 2016: Journal of Cutaneous Pathology
https://www.readbyqxmd.com/read/27910101/early-safety-and-efficacy-of-fingolimod-treatment-in-denmark
#4
A Voldsgaard, N Koch-Henriksen, M Magyari, F Sellebjerg, P S Sørensen, A B Oturai
BACKGROUND: Initiation of fingolimod treatment is associated with a transient decrease of heart rate, and atrioventricular (AV) conduction block may occur. OBJECTIVE: To evaluate the therapeutic effect and safety of fingolimod treatment in MS patients in Denmark with focus on cardiac and pulmonary side effects at treatment onset. MATERIALS & METHODS: We analysed data from the first 496 fingolimod-treated Danish patients, observed for at least 3 months...
January 2017: Acta Neurologica Scandinavica
https://www.readbyqxmd.com/read/27909987/orexin-ox2-receptor-antagonists-as-sleep-aids
#5
Laura H Jacobson, Sui Chen, Sanjida Mir, Daniel Hoyer
The discovery of the orexin system represents the single major progress in the sleep field of the last three to four decades. The two orexin peptides and their two receptors play a major role in arousal and sleep/wake cycles. Defects in the orexin system lead to narcolepsy with cataplexy in humans and dogs and can be experimentally reproduced in rodents. At least six orexin receptor antagonists have reached Phase II or Phase III clinical trials in insomnia, five of which are dual orexin receptor antagonists (DORAs) that target both OX1 and OX2 receptors (OX2Rs)...
December 2, 2016: Current Topics in Behavioral Neurosciences
https://www.readbyqxmd.com/read/27909770/the-natural-history-of-takotsubo-syndrome-a-two-year-follow-up-study-with-myocardial-sympathetic-and-perfusion-g-spect-imaging
#6
Stelvio Sestini, Francesco Pestelli, Mario Leoncini, Francesco Bellandi, Christian Mazzeo, Luigi Mansi, Ignasi Carrio, Antonio Castagnoli
PURPOSE: To investigate changes in sympathetic activity, perfusion, and left ventricular (LV) functionality in takotsubo cardiomyopathy (TTC) patients from onset (T0) to post-onset conditions at 1 month (T1), 1-2 years (T2, T3). METHODS: Twenty-two patients (70 ± 11 years) underwent serial gated single photon emission tomography (G-SPECT) studies with (123)I-mIBG and (99m)Tc-Sestamibi. Statistics were performed using ANOVA/Sheffé post-hoc, correlation test, and receiver operating characteristic (ROC) curve analysis (p < 0...
December 1, 2016: European Journal of Nuclear Medicine and Molecular Imaging
https://www.readbyqxmd.com/read/27909494/anemia-an-independent-predictor-of-adverse-outcomes-in-older-patients-with-atrial-fibrillation
#7
REVIEW
Ali N Ali MBChB MSc Mrcp Uk, Nandkishor V Athavale MBChB Md Frcp, Ahmed H Abdelhafiz MBChB MSc Md Frcp
Both anemia and atrial fibrillation are common in older people and their prevalence is age dependent which increases as population ages. Anemia, especially acute onset, predisposes to new onset atrial fibrillation which is likely to be mediated through inducing heart failure first and this predisposition seems to be potentiated by the presence of renal impairment. Anemia adds to the comorbidity burden of patients with atrial fibrillation and independently increases the risks of adverse outcomes such as increased hospitalization, mortality, bleeding and thromboembolic events...
April 2016: Journal of Atrial Fibrillation
https://www.readbyqxmd.com/read/27909265/prevention-of-the-collapse-of-pial-collaterals-by-remote-ischemic-perconditioning-during-acute-ischemic-stroke
#8
Junqiang Ma, Yonglie Ma, Bin Dong, Mischa V Bandet, Ashfaq Shuaib, Ian R Winship
Collateral circulation is a key variable determining prognosis and response to recanalization therapy during acute ischemic stroke. Remote ischemic perconditioning (RIPerC) involves inducing peripheral ischemia (typically in the limbs) during stroke and may reduce perfusion deficits and brain damage due to cerebral ischemia. In this study, we directly investigated pial collateral flow augmentation due to RIPerC during distal middle cerebral artery occlusion (MCAo) in rats. Blood flow through pial collaterals between the anterior cerebral artery (ACA) and the MCA was assessed in male Sprague Dawley rats using in vivo laser speckle contrast imaging (LSCI) and two photon laser scanning microscopy (TPLSM) during distal MCAo...
December 1, 2016: Journal of Cerebral Blood Flow and Metabolism
https://www.readbyqxmd.com/read/27909226/unique-cytokine-and-chemokine-responses-to-exertional-heat-stroke-in-mice
#9
Michelle A King, Lisa R Leon, Deborah A Morse, Thomas L Clanton
In heat stroke, cytokines are believed to play important roles in multi-organ dysfunction and recovery of damaged tissue. The time course of the cytokine response is well defined in passive heat stroke (PHS), but little is known about exertional heat stroke (EHS). In this study we used a recently developed mouse EHS model to measure the responses of circulating cytokines/chemokines and cytokine gene expression in muscle. A very rapid increase in circulating IL-6 was observed at max core temperature (Tc,max) that peaked at 0...
December 1, 2016: Journal of Applied Physiology
https://www.readbyqxmd.com/read/27909202/prediction-of-blood-brain-barrier-disruption-and-intracerebral-hemorrhagic-infarction-using-arterial-spin-labeling-magnetic-resonance-imaging
#10
Takeya Niibo, Hajime Ohta, Shirou Miyata, Ichiro Ikushima, Kazuchika Yonenaga, Hideo Takeshima
BACKGROUND AND PURPOSE: Arterial spin-labeling magnetic resonance imaging is sensitive for detecting hyperemic lesions (HLs) in patients with acute ischemic stroke. We evaluated whether HLs could predict blood-brain barrier (BBB) disruption and hemorrhagic transformation (HT) in acute ischemic stroke patients. METHODS: In a retrospective study, arterial spin-labeling was performed within 6 hours of symptom onset before revascularization treatment in 25 patients with anterior circulation large vessel occlusion on baseline magnetic resonance angiography...
December 1, 2016: Stroke; a Journal of Cerebral Circulation
https://www.readbyqxmd.com/read/27909129/metallosis-following-implantation-of-magnetically-controlled-growing-rods-in-the-treatment-of-scoliosis-a-case-series
#11
K H Teoh, C von Ruhland, S L Evans, S H James, A Jones, J Howes, P R Davies, S Ahuja
AIMS: We present a case series of five patients who had revision surgery following magnetic controlled growing rods (MGCR) for early onset scoliosis. Metallosis was found during revision in four out of five patients and we postulated a mechanism for rod failure based on retrieval analysis. PATIENTS AND METHODS: Retrieval analysis was performed on the seven explanted rods. The mean duration of MCGR from implantation to revision was 35 months (17 to 46). The mean age at revision was 12 years (7 to 15; four boys, one girl)...
December 2016: Bone & Joint Journal
https://www.readbyqxmd.com/read/27909001/synaptic-phospholipid-signaling-modulates-axon-outgrowth-via-glutamate-dependent-ca2-mediated-molecular-pathways
#12
Johannes Vogt, Sergei Kirischuk, Petr Unichenko, Leslie Schlüter, Assunta Pelosi, Heiko Endle, Jenq-Wei Yang, Nikolai Schmarowski, Jin Cheng, Carine Thalman, Ulf Strauss, Alexey Prokudin, B Suman Bharati, Junken Aoki, Jerold Chun, Beat Lutz, Heiko J Luhmann, Robert Nitsch
Altered synaptic bioactive lipid signaling has been recently shown to augment neuronal excitation in the hippocampus of adult animals by activation of presynaptic LPA2-receptors leading to increased presynaptic glutamate release. Here, we show that this results in higher postsynaptic Ca(2+) levels and in premature onset of spontaneous neuronal activity in the developing entorhinal cortex. Interestingly, increased synchronized neuronal activity led to reduced axon growth velocity of entorhinal neurons which project via the perforant path to the hippocampus...
November 30, 2016: Cerebral Cortex
https://www.readbyqxmd.com/read/27908895/childhood-maltreatment-and-characteristics-of-adult-depression-meta-analysis
#13
REVIEW
Janna Nelson, Anne Klumparendt, Philipp Doebler, Thomas Ehring
BACKGROUND: Childhood maltreatment has been discussed as a risk factor for the development and maintenance of depression. AIMS: To examine the relationship between childhood maltreatment and adult depression with regard to depression incidence, severity, age at onset, course of illness and treatment response. METHOD: We conducted meta-analyses of original articles reporting an association between childhood maltreatment and depression outcomes in adult populations...
December 1, 2016: British Journal of Psychiatry: the Journal of Mental Science
https://www.readbyqxmd.com/read/27908726/activation-of-g-protein-coupled-estrogen-receptor-1-improves-early-onset-cognitive-impairment-via-pi3k-akt-pathway-in-rats-with-traumatic-brain-injury
#14
Ze-Fen Wang, Zhi-Yong Pan, Cheng-Shi Xu, Zhi-Qiang Li
Previous studies experimentally reveal that G-protein coupled estrogen receptor 1(GPER) has neuroprotection against ischemic injury. However, its effect on traumatic brain injury (TBI) is less well-established. Cognitive impairment following human TBI is a common clinical observation, and TBI is considered as a risk factor for Alzheimer's disease (AD). This study aimed to observe the possible protective effect of GPER on early-onset cognitive impairment after a single TBI and investigate the cellular mechanism underlying its actions...
November 28, 2016: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/27908312/calcinosis-in-poly-dermatomyositis-clinical-and-laboratory-predictors-and-treatment-options
#15
Micaela Fredi, Francesca Bartoli, Ilaria Cavazzana, Angela Cerebelli, Nice Carabellese, Angela Tincani, Minoru Satoh, Franco Franceschini
OBJECTIVES: We aimed to identify the possible clinical and laboratory predictors of calcinosis in a cohort of patients with a diagnosis of polymyositis (PM) and dermatomyositis (DM). METHODS: We carried out a retrospective analysis of a cohort of myositis patients attending our clinic between January 2013 and May 2014. RESULTS: 74 patients (58 females, 16 males) with PM (30 cases), DM (30 cases), overlap syndrome (13 cases) and inclusion body myositis (1 case) were enrolled...
November 14, 2016: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/27907920/aneuploidy-detection-and-mtdna-quantification-in-bovine-embryos-with-different-cleavage-onset-using-a-next-generation-sequencing-based-protocol
#16
Miroslav Hornak, David Kubicek, Petr Broz, Pavlina Hulinska, Katerina Hanzalova, Darren Griffin, Marie Machatkova, Jiri Rubes
Bovine embryos are now routinely used in agricultural systems as a means of disseminating superior genetics worldwide, ultimately with the aim of feeding an ever-growing population. Further investigations, common for human IVF embryos, thus have priority to improve cattle IVF, as has screening for aneuploidy (abnormal chromosome number). Although the incidence and consequences of aneuploidy are well documented in human preimplantation embryos, they are less well known for the embryos of other animals. To address this, we assessed aneuploidy levels in thirty-one 2-cell bovine embryos derived from early- and late-cleaving zygotes...
December 2, 2016: Cytogenetic and Genome Research
https://www.readbyqxmd.com/read/27907913/screening-younger-individuals-in-a-primary-care-setting-flags-putative-dementia-cases-and-correlates-gastrointestinal-diseases-with-poor-cognitive-performance
#17
Ilka M Rosa, Ana Gabriela Henriques, Liliana Carvalho, Joana Oliveira, Odete A B da Cruz E Silva
BACKGROUND/AIMS: Diagnosing dementia is challenging in many primary care settings, given the limited human resources and the lack of current diagnostic tools. With this in mind, a primary care-based cohort was established in the Aveiro district of Portugal. METHODS: A total of 568 participants were evaluated using cognitive tests and APOE genotyping. RESULTS: The findings revealed a dementia prevalence of 12%. A strong correlation between increasing Clinical Dementia Rating (CDR) scores and education was clearly evident...
December 2, 2016: Dementia and Geriatric Cognitive Disorders
https://www.readbyqxmd.com/read/27906936/analgesic-effect-of-nalbuphine-when-added-to-intravenous-regional-anesthesia-a-randomized-control-trial
#18
Mohamed Hassan Bakri, Eman Ahmed Ismail, Sayed Kaoud Abd-Elshafy
BACKGROUND: Different adjuvant drugs are currently added to lidocaine for intravenous regional anesthesia (IVRA) to decrease tourniquet and postoperative pain. OBJECTIVE: The aim of the study was to examine the effect of nalbuphine when added toIVRA. STUDY DESIGN: Prospective, randomized, double-blind, controlled clinical trial. SETTING: Assiut University Hospitals. METHODS: One hundred-six adult patients scheduled for unilateral hand surgery under IVRA were randomized into 2 equal groups...
November 2016: Pain Physician
https://www.readbyqxmd.com/read/27906797/non-cirrhotic-portal-fibrosis-in-pediatric-population
#19
Vikrant Sood, Bikrant Bihari Lal, Rajeev Khanna, Dinesh Rawat, Chhagan Bihari Sharma, Seema Alam
BACKGROUND: Non-cirrhotic portal fibrosis (NCPF) has been classically described as a disease of young to middle age with limited literature regarding its occurrence, onset or clinical presentation in children. We hereby present a series of 19 patients diagnosed and managed as NCPF in pediatric age group. METHODS: A retrospective review of all the patients presenting to the pediatric hepatology department (age < 18 years) and diagnosed as NCPF was done and data was evaluated...
November 30, 2016: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/27906487/neutrophil-activation-during-acute-human-anaphylaxis-analysis-of-mpo-and-scd62l
#20
Abbie Francis, Erika Bosio, Shelley F Stone, Daniel M Fatovich, Glenn Arendts, Yusuf Nagree, Stephen P J Macdonald, Hugh Mitenko, Mani Rajee, Sally Burrows, Simon G A Brown
BACKGROUND: The mechanisms involved in the amplification of the mast cell response during anaphylaxis are unclear. Mouse models of anaphylaxis demonstrate the critical involvement of neutrophils. These innate immune cells are highly abundant in peripheral blood and can be rapidly activated to trigger both local and systemic inflammation. OBJECTIVE: To investigate neutrophil activation in peripheral blood during acute human anaphylaxis. METHODS: Patients presenting to the Emergency Department with anaphylaxis underwent blood sampling upon enrolment and at up to three subsequent time points...
December 1, 2016: Clinical and Experimental Allergy: Journal of the British Society for Allergy and Clinical Immunology
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